Hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome

What is Hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome?

Hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome is an extremely rare ectodermal dysplasia syndrome characterized by hypotrichosis universalis with mild to severe scarring alopecia, acro-osteolysis, onychogryphosis, thin and tapered fingertips, periodontitis and caries leading to premature teeth loss, linear or reticular palmoplantar keratoderma and erythematous, scaling, psoriasis-like skin lesions on arms and legs. Lingua plicata and ventricular tachycardia have also been observed.

Other condition names

  • HOPP syndrome
  • Hypotrichosis-osteolysis-periodontitis-palmoplantar hyperkeratosis syndrome
  • Hypotrichosis-striate palmoplantar hyperkeratosis-acroosteolysis-periodontitis syndrome
  • Hypotrichosis-striate palmoplantar keratoderma-acroosteolysis-periodontitis syndrome

Prevalence

  • Worldwide: <1 in 1,000 000

Age of Onset

  • Infancy
  • Neonatal
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